Angelman syndrome

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  • In 1/12,000- 1/25,000 children.
  • Genetic disorder, loss of function in UBE3A gene on chromosome 15.
  • Clinical features apparent after birth, usually within age of 1 or 2 years old.


  • Delayed psychomotor development.
  • In early years often smiling: ‘Happy puppet syndrome’.
  • Overeating.
  • Sleep disturbances.
  • Microcephaly, large tongue and hypersalivation.
  • 90% of the patients have seizures ; usually tonic or atone seizures, or absences.
  • Vulnerable to infections.


  • Frontal slowing
  • Rhytmic discharges in 2-4Hz predominently in the frontal and/or occipital regions.
  • Occipital discharges can be triggered by closing th eyes.
  • EEG similar in patients wit hand without seizures.

Angelman syndrome in an 8-year-old girl (source) with frontal slowing together with occipital and frontal spikes and slow wave complexes, not synchrone

Angelman syndrome in an 8-year-old girl (source).png EEGpedia.png